title: Recombinant human soluble thrombomodulin is active against hemophagocytic lymphohistiocytosis associated with acquired immunodeficiency syndrome.
abstract: A 39-year-old man was admitted to our hospital to initiate highly active anti-retroviral therapy (HAART) for documented acquired immune deficiency syndrome. The HIV load was 2.5 million copies/mL and the CD4-positive lymphocyte count was only 52 cells/µL at presentation. The HAART regimen consisted of lamivudine and abacavir as the backbone, plus raltegravir and lopinavir/ritonavir as the base. The day after initiating HAART, his body temperature rose to 102.4 °F (39.1 °C), accompanied by elevated levels of liver enzymes, neutropenia, coagulopathies, and an extremely high serum ferritin level, prompting us to suspect hemophagocytic lymphohistiocytosis (HLH) and disseminated intravascular coagulation (DIC). To correct the coagulation abnormalities, recombinant thrombomodulin (rTM) was initiated at 375 U/kg. Surprisingly, fever resolved almost immediately, in parallel with dramatic decreases in serum levels of ferritin and liver enzymes and prompt normalization of coagulopathy with only two doses of rTM. The patient subsequently developed amebiasis, which was successfully treated using metronidazole. In summary, the use of rTM dramatically improved not only DIC, but also HLH, suggesting potent anti-inflammatory effects of the agent. Although further clinical reports and trials are needed, rTM appears to provide an additional therapeutic option in the management of HLH.
authors.affiliation: Department of Hematology and Respiratory Medicine, Kochi University, Nankoku, Kochi, 783-8505, Japan.
authors.forename: SungGi
authors.initials: S
authors.lastname: Chi
authors.forename: Takayuki
authors.initials: T
authors.lastname: Ikezoe
authors.forename: Asako
authors.initials: A
authors.lastname: Takeuchi
authors.forename: Masato
authors.initials: M
authors.lastname: Takaoka
authors.forename: Akihito
authors.initials: A
authors.lastname: Yokoyama
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000150:complications* / Q000188:drug therapy; D000328:Adult; D023241:Antiretroviral Therapy, Highly Active; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use*; D018180:Thrombomodulin / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D018180:Thrombomodulin